The clinical and radiological findings in an infant with severe cloverleaf skull associated with a Pfeiffer syndrome are presented. The skull anomaly resulted in raised intracranial pressure and proptosis of the right eye within several weeks of birth. The child died at 3 months of age after subtotal craniectomy. At autopsy a detailed macroscopical and histological analysis of the skull base was conducted. The timing and value of surgery are discussed as well as the pathology of the cloverleaf skull anomaly.